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Achalasia in Children—Clinical Presentation, Diagnosis, Long-Term Treatment Outcomes, and Quality of Life

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Achalasia in Children—Clinical Presentation, Diagnosis, Long-Term Treatment Outcomes, and Quality of Life Journal of Clinical Medicine Article Achalasia in Children—Clinical Presentation, Diagnosis, Long-Term Treatment Outcomes, and Quality of Life Dorota Jarz˛ebicka 1 , Piotr Czubkowski 1,*, Joanna Sieczkowska-Gołub 1, Jarosław Kierku´s 1 , Adam Kowalski 2, Marek Stefanowicz 2 and Grzegorz Oracz 1 1 Department of Gastroenterology, Hepatology, Feeding Disorders and Pediatrics, The Children’s Memorial Health Institute, 04-730 Warsaw, Poland; [email protected] (D.J.); [email protected] (J.S.-G.); [email protected] (J.K.); [email protected] (G.O.) 2 Department of Pediatric Surgery and Organ Transplantation, The Children’s Memorial Health Institute, 04-730 Warsaw, Poland; [email protected] (A.K.); [email protected] (M.S.) * Correspondence: [email protected] Abstract: Background: In spite of the introduction of peroral endoscopic myotomy (POEM), Heller myotomy (HM) remains the mainstay of treatment and the role of pneumatic dilatation (PD) is being debated. The aim of this study was to present a single-center experience in the diagnostic approach and treatment of esophageal achalasia (EA), including the long-term assessment of the QoL. Methods: Data collection was based on the retrospective analysis of clinical notes and prospective interviews with patients and their parents. Results: The study group consisted of 60 patients with EA (F: 26, M: 34), with a median age of 12.0 (1–17) years at diagnosis. The time from the first symptoms until the diagnosis was 1.0 year (0.5–2.0) and the most common were: regurgitation (91.3%), dysphagia (84.8%), and chest pain (47.8%). The diagnostic approach showed a high sensitivity for barium X-ray Citation: Jarz˛ebicka,D.; follow through, esophageal manometry, and endoscopy. Overall, a long-term good outcome of HM Czubkowski, P.; Sieczkowska-Gołub, was achieved in 27 out of 37 patients (73%) and it was negatively affected by the time between the first J.; Kierku´s,J.; Kowalski, A.; Stefanowicz, M.; Oracz, G. Achalasia symptoms and the diagnosis. Out of the 16 patients who underwent PD before HM, a good outcome in Children—Clinical Presentation, was achieved in 14 patients (87.5%), compared to 13 out of 21 patients (62%) who only underwent HM Diagnosis, Long-Term Treatment (p = 0.22). Concomitant fundoplication was routinely performed, and 18% required post-operative Outcomes, and Quality of Life. J. Clin. endoscopic dilatation. At the end of the 12.1 (0.7–26.6)-year follow up, most patients had a good QoL, Med. 2021, 10, 3917. https:// which significantly corresponded with the treatment outcomes. Conclusions: Patients suspected of doi.org/10.3390/jcm10173917 EA should undergo a thorough clinical evaluation including a manometry, a barium X-ray, and an endoscopy. HM is a safe and effective treatment for achalasia and the outcome is not worsened by a Academic Editor: Matthias Biebl preceding endoscopic PD. In most patients, HM alleviates symptoms, although an impaired QoL is common in long-term follow ups. Received: 18 June 2021 Accepted: 25 August 2021 Keywords: myotomy; dysphagia; endoscopic dilatation; quality of life; Urbach scale; AAA syn- Published: 30 August 2021 drome; POEM Publisher’s Note: MDPI stays neutral with regard to jurisdictional claims in published maps and institutional affil- 1. Introduction iations. Esophageal achalasia (EA) is a rare primary esophageal motor disorder, caused by the degeneration or the lack of ganglion cells in the Auerbachian muscle layer in the lower esophagus. EA is characterized by an increased resting tension and an impaired swallow-induced relaxation of the lower esophageal sphincter (LES), which can cause Copyright: © 2021 by the authors. Licensee MDPI, Basel, Switzerland. ineffective esophageal peristalsis [1,2]. A recent epidemiological analysis of the pediatric This article is an open access article population showed an incidence of 0.18/100,000 per year [3]. The occurrence of EA in very distributed under the terms and young children, especially <5 years of age, is extremely rare. It is also a component of AAA conditions of the Creative Commons syndrome (alacrimia, achalasia, Addison disease), also called Allgrove syndrome, and is Attribution (CC BY) license (https:// caused by a mutation in the AAAS gene located on the chromosome 12q13.8 [4]. The most creativecommons.org/licenses/by/ common symptoms are dysphagia, retrosternal chest pain, regurgitation (or vomiting), 4.0/). and weight loss, but still, the diagnosis is often delayed in time [5]. These symptoms are J. Clin. Med. 2021, 10, 3917. https://doi.org/10.3390/jcm10173917 https://www.mdpi.com/journal/jcm J. Clin. Med. 2021, 10, 3917 2 of 11 included in the Eckardt Scoring Scale, which assesses the severity of symptoms in adults [6]. Treatment for EA aims to reduce the pressure of the LES so as to facilitate swallowing and improve the quality of life (QoL). Pharmacological treatment is ineffective, and there are also significant limitations of endoscopic botulinum toxin (BT) injections or pneumatic dilatation (PD). Surgical Heller myotomy (HM) was considered the gold standard due to its high effectiveness and safety [7]. Over the recent years, peroral endoscopic myotomy (POEM) was introduced as a minimally invasive technique, with a success rate similar to that of HM and with less adverse events [8]. There are reports in the literature suggesting that both techniques may be considered as first-line treatment options. Nevertheless, due to the limited access to POEM in children, reserved for a small number of specialized centers, and the short-term outcome measures, the real efficacy of this technique remains to be established [9]. So as to improve care for patients with EA, the International Society for Diseases of the Esophagus (ISDE) has established guidelines for the management of patients with esophageal achalasia, which outline the best practice in the treatment of EA [10]. The aim of this study was to present a single-center experience in the diagnostic approach and treatment of EA, including the long-term assessment of the QoL. Additionally, we described our experience with AAA syndrome patients. 2. Materials and Methods 2.1. Study Design The study included 60 patients aged <18 years with diagnosed EA (a diagnosis based on clinical presentation, imaging, and functional tests), among them 9 patients with AAA syndrome, all treated in our hospital between 1996 and 2017. Patients with EA who did not undergo esophageal manometry but presented with the typical symptoms, a barium follow-through pattern, endoscopic findings, and the relief of symptoms after treatment, were included in the study. The protocol was approved by the Local Ethics Committee (234/KBE/2015). Data collection was based on the retrospective analysis of clinical notes and prospective interviews with the patients and parents who were asked to fill the questionnaires (in 2017), so as to assess the severity of symptoms: dysphagia (difficulty in swallowing and the feeling of a bite of food being stuck in the esophagus), regurgitation, heartburn, chest pain during swallowing, and coughing or choking during eating. The evaluation of tolerated foods included liquid, pulpy, plain, or ordinary diets with the necessity of drinking during the meals. The severity of symptoms was assessed according to their frequency per week and per month. Families were contacted over the phone and assisted in answering the questions. Based on the collected data, the long-term outcome of the treatment and the QoL were assessed. We did not include retrospective data on the long-term outcome of the treatment from clinical notes due to the quality of data, missing information, and the transition of patients to the other centers for adults. 2.2. Evaluation of Diagnostic Methods All results of the investigations were re-evaluated with a senior radiologist or endo- scopist and categorized according to a unified scoring system, as follows: 1. Barium X-ray follow through was evaluated by one radiologist on a radiological scale of EA according to Rezende et al. [11]: grade I—slow esophagus emptying, peristaltic disorders—tertiary waves or a lack of peristalsis; grade II—a slight enlargement of the esophagus, and more intense tertiary waves; grade III—a significant widening of the esophagus, with a narrowing of the lower segment—a characteristic image of the “bird’s beak”, violent convulsive muscle spasms or a complete lack of peristalsis of the esophagus; grade IV — the image as in stage III and a very large dilation of the esophagus with the change of its axis; 2. Gastroscopy results were evaluated according to the presence of the following: resid- ual food in the esophagus, an enlargement of the esophagus, changes in the mucosa J. Clin. Med. 2021, 10, 3917 3 of 11 of the esophagus (resulting from long-residual food in the esophagus), stomach cardia—sometimes with a resistance passing the endoscope; 3. In the manometry, four basic features of EA were assessed: an increased resting pressure of the LES > 45 mmHg, a lack or incomplete LES relaxation in response to the incoming bite of food (LES > 8 mmHg), a lack of esophageal motility, and the positive pressure in the esophagus. 2.3. Treatment 2.3.1. Endoscopic Pneumatic Dilatation All procedures were performed by two senior endoscopists. In the majority, patients required a prompt intervention due to the high severity of symptoms, and PD was con- sidered as a bridging therapy to HM. Under an X-ray control, the balloon was placed in the LES area and inflated until the narrowing was smoothed. The diameter of the balloon was selected depending on the patient’s age and severity of esophageal stenosis, usually between 15–40 mm. In this position, the balloon was held from 15–20 s to several minutes. During one session, the balloon was inflated 1 or 2 times. After the balloon removal, a routine endoscopic assessment was performed to check for a possible mucosa injury or an esophageal perforation.
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